Health-Related Quality of Life in Adult Patients with Common Variable Immunodeficiency Disorders and Impact of Treatment

Abstract

Purpose: Common variable immunodeficiency disorder (CVID) is a primary immunodeficiency disease (PIDD) often associated with severe and chronic infections. Patients commonly receive immunoglobulin (Ig) treatment to reduce the cycle of recurrent infection and improve physical functioning. However, how Ig treatment in CVID affects quality of life (QOL) has not been thoroughly evaluated. The purpose of a recent Immune Deficiency Foundation (IDF) mail survey was to assess the factors that are associated with QOL in patients with CVID receiving Ig treatment.

Methods: A 75-question survey developed by the IDF and a 12-item Short Form Health Survey (SF-12) to assess QOL were mailed to adults with CVID. Mean SF-12 scores were compared between patients with CVID and the general US adult population normative sample.

Results: Overall, 945 patients with CVID completed the surveys. More than half of the patients (54.9%) received intravenous Ig and 44.9% received subcutaneous Ig treatment. Patients with CVID had significantly lower SF-12 scores compared with the general US population regardless of sex or age (p < 0.05). Route of IgG replacement did not dramatically improve QOL. SF-12 scores were highest in patients with CVID who have well-controlled PIDD, lacked physical impairments, were not bothered by treatment, and received Ig infusions at home.

Conclusion: These data provide insight into what factors are most associated with physical and mental health, which can serve to improve QOL in patients in this population. Improvements in QOL can result from early detection of disease, limiting digestive system disease, attention to fatigue, and implementation of an individual treatment plan for the patient.

Keywords: Primary immunodeficiency diseases; common variable immunodeficiency; mental health; physical health; quality of life; reported health.

Fig. 1

SF-12 mean physical (general health, bodily pain, role–physical, and physical functioning) and mental (vitality, social functioning, role–emotional, and mental health) component and domain scores for adult patients with CVID compared with the US mean score (top) and by sex compared with a general US normative population (bottom). ^* Significant difference between patients with CVID compared with the US mean score (top figure) and with the sex-specific general US normative population sample (bottom figure) (p < 0.05; lower than the US norm). ^† Significant difference between male patients with CVID compared with females with CVID (with the exception of MCS) (p < 0.05). CVID common variable immunodeficiency, SD standard deviation, SF-12 12-item Short Form Health Survey

Fig. 2

SF-12 mean physical and mental component scores for patients with CVID, by age group, compared with a general US normative population. ^* Significant difference between patients with CVID compared with the age-specific general US normative population (p < 0.05 for PCS and MCS; lower than the US norm). CVID common variable immunodeficiency, SD standard deviation, SF-12 12-item Short Form Health Survey

Fig. 3

SF-12 mean physical (general health, bodily pain, role–physical, and physical functioning) and mental (vitality, social functioning, role–emotional, and mental health) component and domain scores for patients with CVID, by permanent loss/impairment (the number of permanent impairments refers to any type of impairment in the survey: digestive, kidney, liver, lung, or neurological function; hearing, mobility, vision, or others not listed), by number of permanent losses/impairments (between-group comparisons), and by severity of limitations in the past 12 months. Note, permanent impairment was patient described through the use of the 75 Question IDF Survey via item number 8. ^* Significant difference between all patients with CVID compared with the US mean score (p < 0.05; lower than the US norm) (top and middle figures) and significant difference between the mean SF-12 in the US normative population and patients with a “slight limitation” or worse (p < 0.05; lower than the US norm) and “no limitation” (p < 0.05; higher than the US norm) (all categories; bottom figure). ^† Significant difference between patients who had “no impairments” compared with patients who had a “permanent impairment” in digestive and/or lung function or between patients with 0–2 and ≥3 impairments (p < 0.05) (top and middle figures) and significant difference between patient groups (“no limitation” vs “slight limitation,” “slight limitation” vs “moderate limitation,” and “moderate limitation” vs “severe limitation”) (p < 0.05) (bottom figure). CVID common variable immunodeficiency, SD standard deviation, SF-12 12-item Short Form Health Survey

Fig. 4

SF-12 mean physical (general health, bodily pain, role–physical, and physical functioning) and mental (vitality, social functioning, role–emotional, and mental health) component scores for patients with CVID, by route of Ig administration (top) and by bother of treatment and route of Ig administration (There were significant between-group differences between “not at all bothered” vs “bothered a little bit” for MCS (p < 0.05); “not at all bothered” vs “moderately bothered” for PCS and MCS (p < 0.05); and “not at all bothered” vs “extremely bothered” for PCS and MCS (p < 0.05). There were no significant differences by type of Ig administration (SCIG vs IVIG)) (bottom), compared with a US normative population. ^* Significant difference between any route of Ig administration (SCIG or IVIG) compared with a US normative population (p < 0.05; lower than the US norm; top figure) and significant difference in mean SF-12 scores between a general US normative population and patients who were “moderately bothered” or worse (PCS and MCS) and “not at all bothered” (PCS) (p < 0.05; lower than the US norm; bottom figure). ^† Significant difference between SCIG and IVIG (p < 0.05). CVID common variable immunodeficiency, Ig immunoglobulin, IVIG intravenous administration of Ig, SCIG subcutaneous administration of Ig, SD standard deviation, SF-12 12-item Short Form Health Survey

Fig. 5

SF-12 mean physical (general health, bodily pain, role–physical, and physical functioning) and mental (vitality, social functioning, role–emotional, and mental health) component scores for patients with CVID, by route of Ig administration (There were no significant differences in mean SF-12 scores between SCIG or IVIG; between-group comparisons indicated a significant difference in SF-12 scores between patients who reported “never” experiencing postinfusion fatigue and patients who reported “always” or “occasionally” experiencing postinfusion fatigue (p < 0.05)) (“Does the patient experience periods of fatigue or low energy between Ig treatments?”). ^* Significant difference between patients who “never” experience periods of fatigue or low energy compared with the general US population normative sample (p < 0.05) (lower PCS than the US norm and higher MCS than the US norm). ^† Significant difference between patients who “occasionally” and “always” experience periods of fatigue or low energy compared with a US normative population (p < 0.05) (lower PCS and MCS). CVID common variable immunodeficiency, Ig immunoglobulin, IVIG intravenous administration of Ig, MCS mental component score, PCS physical component score, SCIG subcutaneous administration of Ig, SD standard deviation, SF-12 12-item Short Form Health Survey

Fig. 6

SF-12 mean physical (general health, bodily pain, role–physical, and physical functioning) and mental (vitality, social functioning, role–emotional, and mental health) component and domain scores for patients with CVID, by Ig treatment location (includes doctor’s private office (n = 31), hospital clinic (n = 26), and other (n = 12)) (“Where does the patient usually receive Ig therapy?”). ^* Significant difference between patients who received IVIG at home vs at an infusion suite (p < 0.05). ^† Significant difference between all patients with CVID compared with the US mean score (p < 0.05; lower than the US norm). CVID common variable immunodeficiency, Ig immunoglobulin, IVIG intravenous administration of Ig, SCIG subcutaneous administration of Ig, SD standard deviation, SF-12 12-item Short Form Health Survey